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1 Department of Biochemistry, Erasmus Medical Center, Rotterdam, The Netherlands
2 Department of Pediatrics, Sophia Children's Hospital, Rotterdam, The Netherlands
3 Department of Pediatrics, Academic Hospital, Groningen, The Netherlands
* To whom correspondence should be addressed. E-mail: m.bijvelds{at}erasmusmc.nl.
Cystic fibrosis (CF) is frequently associated with progressive loss of exocrine pancreas function,
leading to incomplete digestion and absorption of dietary fat. Supplementing patients with
pancreatic lipase reduces fat excretion, but it does not completely correct fat malabsorption,
indicating that additional pathological processes affect lipolysis and/or uptake of lipolytic
products. To delineate the role of such (post)lipolytic processes in CF-related fat malabsorption,
fat absorption, lipolysis and fatty acid uptake were assessed in two murine CF models by
measuring fecal fat excretion and uptake of oleate- and triolein-derived lipid. Pancreatic and
biliary function was investigated by determining lipase secretion and biliary bile salt (BS)
secretion, respectively. A marked increase in fecal fat excretion was observed in cftr null mice,
but not in homozygous 
F508 mice. Fecal BS loss was enhanced in both CF models, but biliary
BS secretion rates were similar. Uptake of free fatty acid was delayed in both CF models, but
only in null mice a specific reduction in lipolytic activity was apparent, characterized by strongly
reduced triglyceride absorption. Impaired lipolysis was not due to reduced pancreatic lipase
secretion. Suppression of gastric acid secretion partially restored lipolytic activity and lipid
uptake, indicating that incomplete neutralization of gastric acid impedes fat absorption. We
conclude that fat malabsorption in cftr null mice is caused by impairment of lipolysis, which may
result from aberrant duodenal pH regulation.
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